Idiopathic Arterial Calcification: Experience from a Single Center in South India

Abstract

Idiopathic arterial calcification of infancy is a rare autosomal recessive disease, characterized by deposition of calcium along the internal elastic membrane of arteries, accompanied by fibrous thickening of the intima which causes luminal narrowing. We hereby report a series of 20 cases seen over a period of 10 years. Nineteen cases were reported as idiopathic arterial calcification and one was reported as arterial calcification in a twin reversed arterial perfusion sequence. The sites of calcification were mainly aortic valve and root of aorta which was involved in 100% of cases followed by pulmonary artery up to its branches in 18 (90%) cases. There was recurrence in the subsequent pregnancies in three of the cases. Genetic association with ENPP1 was proved in one case. There was no survival in all the cases. Thus, the prognosis of idiopathic arterial calcification is very poor with very few long-term survivors.